Phenyl alpha-L-iduronide cyclohexylammonium saltA substrate for alpha-L-iduronidase

Phenyl α-L-iduronide cyclohexylammonium salt (CAS 39031-70-4)

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Application: A substrate for alpha-L-iduronidase
CAS Number: 39031-70-4
Molecular Weight: 369.41
Molecular Formula: C12H14O7•C6H13N
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Phenyl α-L-iduronide cyclohexylammonium salt is used as an enzyme substrate. The activities of alpha-L-iduronidase (EC 3.2.1.76) have been measured using phenyl-α-L-iduronide as the substrate. Measurement of α-L-iduronidase activity using phenyl α-L-iduronide may enable a reliable diagnosis for Hurler syndrome to be made within 15 to 18 days after amniocentesis. Recent studies suggest that 4-Methylumbelliferyl α-L-iduronide (sc-262101) may provide a more sensitive assay for the estimation of α-l-iduronidase in cultured cells and could be used to diagnose Hurler's disease.


References

1. Butterworth, J. and Broadhead, D.M. 1980. J. Inherit. Metab. Dis. 2(3): 71-74. PMID: 6796763

2. Kleijer, W.J. et al. 1981. Clin. Chim. Acta. 116(1): 47-54. PMID: 6797759

3. Kleijer, W.J. et al. 1983. Prenat. Diagn. 3(3): 179-186. PMID: 6413967

Physical State :
Solid
Storage :
Store at -20° C
For Research Use Only. Not Intended for Diagnostic or Therapeutic Use.
PubChem CID :
53398999
SMILES :
C1CCC(CC1)N.C1=CC=C(C=C1)OC2C(C(C(C(O2)C(=O)O)O)O)O

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